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Movement Disorders in Children

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Title: Movement Disorders in Children


1
Movement Disorders in Children
2
Overview
  • Childhood movement disorders occur secondary to a
    wide range of genetic and acquired disorders
    affecting brain development. 
  •  Classification by type of abnormal movement
  • Bradykinetic disorders
  • Hyperkinetic disorders
  • Classification by Etiology
  • Primary
  • Secondary
  • Fixed Structural lesion
  • Degenerative
  • Metabolic
  • Drug Induced
  • Infectious
  • Important point
  • Any disorder that affects the basal ganglia can
    cause a wide array of different movement
    disorders
  • Static brain injury may nonetheless cause a
    changing movement disorder, as development and
    brain plasticity alter the brains response to
    injury.

3
Basal Ganglia
  • Group of deep nuclei
  • Caudate nucleus
  • Putamen
  • Globus pallidus
  • Substantia nigra
  • dopamine-rich pars compacta
  • pars reticularis
  • Inputs Corpus striatum (caudate nucleus and
    putamen) receives input from the cerebral cortex
    and the thalamus
  • Outputs projects by way of the thalamus to the
    cerebral cortex and then to the pyramidal system

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Bradykinetic vs. Hyperkinetic
  • Bradykinetic disorders
  • Very rare in children
  • Parkinson disease is the most common bradykinetic
    disorder
  • Hyperkinetic disorders
  • Tic Disorders
  • Dystonia
  • Sterotypies
  • Chorea
  • Athetoses
  • Ballismus
  • Tremor
  • Myoclonus
  • Dyskinesia

8
Tic Disorders
  • Tics are repeated, intermittent movements that
    are almost always briefly suppressible and are
    usually associated with awareness of an urge to
    perform the movement
  • Tourette syndrome Multiple motor and vocal tics
  • Etiology
  • Primary
  • the vast majority
  • Secondary
  • Huntingtons, encephalitis, medication induced,
    carbon monoxide poisoning, neuroacantocytosis

9
Tics
  • Diagnosis
  • Typical movements
  • Dont occur in sleep
  • Patient usually unaware of it occurring
  • Patient can usually suppress for a short time
  • But when they do, it is accompanied by a
    discomfort and a strong urge to do the tic (a
    compulsion)
  • Wax and wane over time
  • Worsen with stress
  • Associated with ADHD and OCD
  • Make sure to ask both of patient and family
    history
  • PANDAS
  • Treatment
  • Reassurance
  • Tics tend to wax and wane most children outgrow
    them
  • Medications (when necessary)
  • Stimulants bring out tics if the have ADHD, they
    cant use stimulants
  • Tenex
  • Risperidone

10
Dystonia
  • Involuntary sustained or intermittent muscle
    contractions that cause twisting and repetitive
    movements, abnormal postures, or both.

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Dystonia
13
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Classification of Dystonia
  • By location
  • Generalized dystonia affects most or all of the
    body.
  • Focal dystonia is localized to a specific part of
    the body.
  • Blepharospasm, Cervical Dystonia, Task Specific
    Dystonia (eg Writers cramp)
  • Multifocal dystonia involves two or more
    unrelated body parts.
  • Segmental dystonia affects two or more adjacent
    parts of the body.
  • Hemidystonia involves the arm and leg on the same
    side of the body.
  • By etiology
  • Primary by definition, no other neurologic
    impairment
  • Secondary Cerebral Palsy the most common cause
    in children
  • Psychogenic

16
Primary Dystonias
  • Genetic Dystonias
  • DYT1 dystonia 
  • dominantly inherited generalized dystonia
  • typically begins in childhood, affects the limbs
    first, and progresses
  • A great deal of phenotypic variability
  • Dopa-responsive dystonia (Segawas disease)
  • onset during childhood and have progressive
    difficulty with walking.  
  • Symptoms characteristically fluctuate and are
    worse late in the day and after exercise. 
  • Some forms are due to mutations in the DYT5 gene
    for GTP cyclohydrolase 1.   
  • Patients with this disorder have dramatic
    improvements in symptoms after treatment with
    levodopa
  • Many other genes that cause dystonic syndromes
    have been found

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Secondary Dystonias
  • Fixed Injury/Structural
  • Kernicterus,, head trauma, encephalitis tumors,
    stroke, congenital malformations
  • Degenerative Fahr's disease ,
    pantothenate-kinase associate dneurodegenerative
    disease (Hallervorden-Spatz disease),
    Huntington's disease, spinocerebellar ataxias
    neuronal ceroid lipofuscinosis Rett syndrome
    Tay-Sachs disease Sandhoff's disease
    Niemann-Pick type C metachromatic
    leukodystrophy Leigh's disease
    neuroacanthocytosisPelizaeus-Merzbacher
    disease ataxia-telangiectasia
  • Chemical/Metabolic Glutaric aciduria
    mitochondrial disorders Wilson's disease
    homocystinuria Lesch-Nyhan disease
    methylmalonic aciduria tyrosinemia, vitamin E
    deficiency
  • Drug- or Toxin-induced
  • Neuroleptic and anti-emetic medications (e.g.,
    haloperidol, thorazine, olanzapine, risperidone,
    quetiapine, compazine, prochlorperazine,
    metoclopramide, etc.) calcium channel blockers
    stimulants , anticonvulsants (e.g.,
    carbamazepine, phenytoin, ) thallium manganese
    carbon monoxide ethylene glycol cyanide
    methanol wasp sting
  • Paroxysmal
  • Paroxysmal kinesogenic choreoathetosis familial
    periodic paralysis complex migraine alternating
    hemiplegia paroxysmal torticollis of infancy
  • Psychogenic
  • Disorders That Mimic Dystonia
  • Tonic seizures, syringomyelia Arnold-Chiari
    malformation type II posterior fossa mass
    cervical spine malformation, Sandifer's syndrome
    spasmus nutans tics self-stimulation
    spasticity myotonia hyperexplexia disorders

19
Work up of Dystonia
  • Take careful history of medication, drug and
    supplement use
  • Consider Genetic testing (especially DYT1)
  • Consider empiric trial of levodopa
  • Consider metabolic testing amino acids, organic
    acis, Wilsons testing, lysosomal storage diseases
  • Consider MRI

20
Treatment of Dystonia
  • Botulinum toxin
  • Particularly for focal dystonias
  • Medications
  • Anticholinergic agents trihexyphenidyl and
    benztropine.
  • GABAergic agents  benzodiazepines, baclofen
  • Dopaminergic agents tetrabenazine
  • Levodopa for Dopa-responsive dystonia (DRD)
  • Deep brain stimulation (DBS)
  • Physical and other therapies

21
Sterotypies
  • Repetitive, simple movements that can be
    voluntarily suppressed.
  • Examples include repetitive chewing, rocking,
    twirling, or touching movements
  • Most common in children with autism or mental
    retardation can occur in otherwise normal
    children.

22
Rett Syndrome
23
Chorea, athetosis and ballismus
  • Chorea
  • an irregular, rapid, uncontrolled, involuntary,
    excessive movement that seems to flow randomly
    from one part of the body to another.
  • The affected child often appears fidgety or
    restless and cant sit still
  • Athetosis
  • A slower writhing and twisting movement.
  • Ballism (ballismus)
  • chorea that affects proximal joints such as
    shoulder or hip, leading to large amplitude
    flailing movements of the limbs

24
Etiology of Chorea
  • Fixed injury/Structural
  • Cerebral Palsy (Kernicterus), Tumor, Trauma,
    Stroke
  • Degenerative
  • Huntingtons, Neuroacanthocytosis,
    Ataxia-telangiectasia Fahr's disease
    pantothenate kinase 2 deficiency
    ("Hallervorden-Spatz disease") Rett syndrome
    Niemann-Pick disease type C , Pelizaeus-Merzbacher
    disease GM1 gangliosidosis, metachromatic
    leukodystrophy Wilson's disease ,etc
  • Drug Induced/Toxins
  • Neuroleptics, antiparkinson drugs, tricyclics ,
    amphetamines, anticonvulsants, anticholinergics
  • Metabolic
  • Hyperthyroid, hypoparathyroid, pregnancy, hyper
    and hypo natremia, hypomagnesemia, hypoclaciemia,
    nutrional deficiencies (beriberi, pellagra, B12
    deficiency)
  • Acyl-CoA dehydrogenase deficiency Lesch-Nyhan
    disease methylmalonic aciduria vitamin E
    deficiency propionic acidemia
  • Infectious
  • Sydenhams chorea
  • Immunological
  • SLE, HSP
  • Migraine
  • Psychogenic

25
Kernicterus
26
Sydenham chorea
  • Sydenham chorea is a movement disorder
    characterized by chorea, emotional lability, and
    hypotonia. It is one of the major clinical
    manifestations of acute rheumatic fever (ARF).
  • Symptoms of SC usually begin one to eight months
    after the onset of ARF. The symptoms typically
    improve gradually, with a mean duration of 12 to
    15 weeks (
  • At least 30 percent of individuals have clinical
    carditis in association with SC.
  • Diagnosis
  • The diagnosis of SC is made clinically, based on
    characteristic neurological findings and a
    careful cardiac examination. If carditis is
    present, this confirms the diagnosis.
  • The antistreptolysin O (ASLO) titer is of limited
    use in patients with SC, because titers generally
    peak before the onset of SC symptoms and children
    without rheumatic fever or SC often have low
    positive titers of ASLO.
  • The antideoxyribonuclease (anti-DNAse) B titer is
    more useful for supporting the diagnosis of SC
    because it tends to remain elevated longer.
  • If not clinicually definite, other causes of
    chorea should be excluded, including systemic
    lupus erythematosus, Huntingtons disease, and
    Wilsons disease.
  • Treatment
  • Most patients with SC recover fully without
    treatment, with symptoms lasting from a few weeks
    to one year or more.
  • For those with significant impairment of motor
    function and the possibility of self injury
    consider corticosteroids (prednisone 1 mg/kg
    daily for two weeks and then tapered over two to
    three weeks)
  • Valproic acid if needed to treat chorea
  • Up to 30 percent of individuals with SC
    experience a recurrence, usually within a few
    years of the initial episode. The risk is
    probably reduced, by chronic treatment with
    prophylactic antibiotics.

27
Sydenham Chorea
28
Athetosis
  • http//www.youtube.com/watch?vgNKKZAfMr8M

29
Work up
  • Take careful history of medication, drug and
    supplement use
  • If acute onset throat culture and streptococcal
    blood antigen test (ASLO, anti-DNAse),
    electrolytes, magnesium, calcium, thyroid
    function, CBC
  • Consider amino and organic acid studies, ammonia,
    antinuclear antigen (ANA), antiphospholipid
    antibodies (APLA), work up for Wilsons disease
    (start with ceruloplasmin), evaluation of CBC for
    acanthocytes.
  • Consider MRI

30
Treatment of Chorea
  • May be difficult to treat.
  • Taper or discontinue any medications that can
    cause or worsen chorea
  • In adults, the mainstay of treatment in adults is
    neuroleptics, including haloperidol and pimozide.
  • In children the incidence of side effects in
    children is high.
  • Therefore, treatment is usually
  • Benzodiazepine, particularly clonazepam,
    diazepam, or clobazam
  • Valproate, especially in Sydenham's chorea.
  • Sydenham's chorea
  • There is considerable debate about whether
    children with Sydenham's chorea due to
    streptococcal infection should be given long-term
    antibiotics. There is not yet scientific evidence
    to support this, although short-term treatment is
    certainly needed in order to prevent
    complications such as rheumatic fever.

31
Tremor
  • A rhythmic back-and-forth or oscillating
    involuntary movement about a joint.

32
Classification of Tremor
  • Classification by type of tremor
  • Rest Tremor
  • Parkinsons, Wilson Disease, Severe essential
    tremor
  • Action Tremor
  • Postural
  • Kinetic
  • Intention Cerebellar Tremor
  • Task Specific
  • Isomeric
  • Classification by Etiology
  • Physiologic tremor
  • Essential tremor
  • Associated w/ Peripheral Neuropathy Charcot
    MarieTooth
  • Psychogenic

33
Etiology of Tremor
  • Primary Tremors
  • Enhanced physiologic tremor
  • Essential Tremor
  • Static (fixed) injuryStroke (particularly in
    the midbrain or cerebellum) multiple sclerosis
  • Degenerative
  • Juvenile parkinsonism Wilson's disease
    Huntington's disease Tay-Sachs disease
  • Chemical/metabolic
  • Hyperthyroidism hyper-adrenaline state
    (including anxiety or pheochromocytoma)
    hypomagnesemia hypocalcemia hypoglycemia
    hepatic encephalopathy
  • Drug-induced
  • Valproate lithium thyroid hormone albuterol,
    tricyclic antidepressants stimulants,
    neuroleptics cyclosporine mercury thallium
    nicotine lead manganese arsenic cyanide
    ethanol
  • Psychogenic tremor
  • Other causes of tremor
  • Peripheral neuropathy, cerebellar disease or
    malformation,spasmus nutans

34
Essential Tremor
  • Tremor should be the only neurologic
    manifestation
  • Usually benign, but may progress to a disabling
    movement disorder.
  • Hereditary ET can begin in infancy
  • hereditary chin tremor and shuddering attacks.

35
Work up of Tremor
  • Any medications that may worsen tremor should be
    avoided, if possible.
  • Check electrolytes, including glucose, calcium
    and magnesium, thyroid function, copper in the
    urine (for Wilson's disease), and possibly the
    amount of adrenaline metabolites (for
    pheochromocytoma).
  • Consider MRI if the tremor had sudden onset,
  • Consider EEG if there is suspicion for seizures.
  • If parkinsonian features are present, consider a
    trial of L-DOPA
  • If there is a family history of tremor, it may be
    helpful to of alcohol ( in the affected family
    member). This suggests essential tremor.

36
Treatment of Tremor
  • Often, mild tremor does not require treatment.
  • Medications
  • Propranolol
  • Primidone
  • benzodiazepines (i.e., clonazepam, diazepam,
    lorazepam).

37
Myoclonus
  • Sudden, brief, jerky, shock-like involuntary
    movements.
  • May be triggered by attempts at voluntary
    movement, sensory stimulation or startle
  • Myoclonus is not suppressible and is often
    activated by volitional movement.
  • Negative myoclonus is a sudden involuntary
    relaxation of a muscle, rather than a
    contraction.
  • Myoclonus is often associated with epilepsy. 

38
Classification and Etiology of Myoclonus
  • Physiological e.g., sleep myoclonus, benign
    myoclonus of infancy
  • Essential Myoclonus familial essential
    myoclonus, essential myoclonus-dystonia,
    stimulus-sensitive myoclonus
  • Epileptic e.g., juvenile myoclonic epilepsy,
    progressive myoclonic epilepsies, epilepsia
    partialis continua, Rasmussen's encephalitis,
    early infantile myoclonic encephalopathy,
    infantile spasms, Lennox-Gastaut syndrome, benign
    familiar myoclonic epilepsy, Angelman syndrome
  • Symptomatic
  • Fixed injury e.g., carbon-monoxide poisoning,
    hypoxic injury or near-drowning, heatstroke,
    trauma, stroke, electrocution
  • Storage/Degenerative diseases e.g., sialidoses,
    lipidosis, storage diseases, Wilson's disease,
    Rett syndrome, mitochondrial disorders,
    spinocerebellar ataxias
  • Infections/Para-infectious e.g., Creutzfeldt-Jaco
    b disease, steptococcus, viral encephalitis
  • Endocrine e.g., hyperthyroidism, hyponatremia,
    hypoglycemia
  • Structural e.g., tumors that irritate brain in
    direct manner or release chemicals into the blood
  • Drug-induced/Toxins e.g., anti-seizure
    medications, antidepressants, stimulants,
    liver-toxic medications, respiratory depressants,
    corticosteroids, acyclovir, L-dopa
  • Associated with systemic illness e.g., dialysis,
    renal failure, liver failure, pulmonary disease,
    carbon dioxide intoxication

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